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      Pazopanib-mediated Long-term Disease Stabilization after Local Recurrence and Distant Metastasis of Primary Intracranial Leiomyosarcoma: A Case Report on the Efficacy of Pazopanib as a Salvage Therapy

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          Abstract

          Primary intracranial leiomyosarcoma (LMS) is an extremely rare tumor of the central nervous system. Only sporadic case reports have been published, and therefore data regarding long-term prognosis remain scarce. A 76-year-old woman presented with a right parietal mass, which had grown rapidly in the month prior to admission. Neuroimaging showed a resemblance to intraosseous meningioma. Gross total resection of the tumor was achieved, and histological diagnosis confirmed LMS. Because positron emission tomography (PET) with fluorodeoxyglucose (FDG) just after the resection showed no abnormal uptake, we diagnosed the tumor as primary intracranial LMS. Follow-up PET at 16 months after treatment showed two foci of FDG uptake in the bilateral lungs. Histological diagnosis by surgical resection identified the lesions as lung metastases of LMS. In addition, follow-up head magnetic resonance imaging (MRI) at 31 months showed local recurrence, and we conducted salvage therapy using CyberKnife system (Accuray incorporated) and pazopanib. To date, for 15 months after local recurrence, she is alive with intracranial recurrent disease remained inactive.

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          Phase II trial of pazopanib (GW786034), an oral multi-targeted angiogenesis inhibitor, for adults with recurrent glioblastoma (North American Brain Tumor Consortium Study 06-02).

          The objective of this phase II single-arm study was to evaluate the efficacy and safety of pazopanib, a multi-targeted tyrosine kinase inhibitor, against vascular endothelial growth factor receptor (VEGFR)-1, -2, and -3, platelet-derived growth factor receptor-alpha and -beta, and c-Kit, in recurrent glioblastoma. Patients with or =6 months (PFS6 = 3%). Thirty patients (86%) had died and median survival was 35 weeks (95% CI: 24-47 weeks). Pazopanib was reasonably well tolerated with a spectrum of toxicities similar to other anti-VEGF/VEGFR agents. Single-agent pazopanib did not prolong PFS in this patient population but showed in situ biological activity as demonstrated by radiographic responses. ClinicalTrials.gov identifier: NCT00459381.
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            Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content.

            Leiomyosarcoma of soft tissue is a rare tumor. There are different opinions regarding epidemiology and prognosis. Epidemiology and prognosis were analyzed in a consecutive, population-based series of 48 patients with subcutaneous and deep-seated leiomyosarcoma in the extremities and trunk wall with a complete follow-up of a minimum of 3 years. Cutaneous tumors were not included. The annual incidence was 0.13/10(5). The ratio of men to women was 1.2, and the median age was 65 years. The thigh was the most common location. Almost half of the tumors were subcutaneous. The median tumor size was 6 cm (range, 1-25 cm). All patients were treated with surgery, and in 19 cases it was combined with adjuvant radiation therapy or chemotherapy. The cumulative 5-year survival rate was 64%. Multivariate analysis indicated that age of 60 years or greater (relative risk [RR] = 8) and intratumoral vascular invasion (RR = 4) were independent risk factors for death resulting from tumor. DNA aneuploidy (RR = 4) and tumor necrosis (RR = 3) were associated with poor prognosis, but did not reach statistic significance. Advanced age, vascular invasion, and DNA aneuploidy could be used to identify prognostic subgroups.
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              Primary intraosseous meningioma: CT and MRI appearance.

              Benign primary intraosseous meningioma presenting with osteolytic skull lesion and soft-tissue component is rare. CT and MR imaging of a patient with frontoparietal scalp swelling showed an osteolytic intracalvarial lesion with an extradural soft-tissue component. Following wide surgical resection, the histological examination revealed an intraosseous chordoid meningioma. The clinical and radiological findings of primary intraosseous meningioma are discussed and the relevant literature is reviewed.
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                Author and article information

                Journal
                NMC Case Rep J
                NMC Case Rep J
                NMCCRJ
                NMC Case Report Journal
                The Japan Neurosurgical Society
                2188-4226
                January 2018
                07 December 2017
                : 5
                : 1
                : 1-7
                Affiliations
                [1 ]Department of Neurosurgery, National Hospital Organization Kyoto Medical Center, Kyoto, Kyoto, Japan
                [2 ]Department of Pathology, National Hospital Organization Kyoto Medical Center, Kyoto, Kyoto, Japan
                [3 ]Department of Medical Oncology, National Hospital Organization Kyoto Medical Center, Kyoto, Kyoto, Japan
                [4 ]Department of Chest Surgery, National Hospital Organization Kyoto Medical Center, Kyoto, Kyoto, Japan
                Author notes
                Corresponding author: Yasuhiro Kawabata, MD, Department of Neurosurgery, National Hospital Organization Kyoto Medical Center, 1-1 Mukaihata-cho, Fukakusa, Fushimi-ku, Kyoto 612-8555, Japan. ykawabata-kyt@ 123456umin.net
                Article
                nmccrj-5-01
                10.2176/nmccrj.cr.2017-0048
                5767479
                29354331
                ff8a0de6-66e2-44a4-b257-2e8a73a1a978
                © 2018 The Japan Neurosurgical Society

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/

                History
                : 22 February 2017
                : 16 May 2017
                Categories
                Case Report

                leiomyosarcoma,lung metastasis,local recurrence,pazopanib

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